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Complaint :
45 days female baby  suffering with fever & intractable convulsions from  last 4 days.

Clinical futures & lab :
 systemic examination is normal.CSF analysis is normal.

Findings :
Sulci are absent with smooth cortical surface. Sylvian fissures are straight oblique resulting

a figure of  eight appearance of brain. Colpocephaly +.

Diagnosis : Lissencephaly.

Discussion :
Lissencephaly or smooth brain  refer to brain with absent or poor sulcation. It is physiological at 16-17 weeks of intrauterine life. Intrauterine infections can also results in asmooth, Lissencephalic -appearing brain.

Lissencephaly is 3  types.

Type 1 shows colpocephaly and a thickened cortex with broad, flat gyri, smooth  white- gray matter interface, and straight oblique or shallow sylvian fissures,giving a figure of eight appearance. Parenchymal calcifications
can be present if Lissencephaly is due intrauterine infections.

Lissencephaly type 11 shows  agyric, severely disorganized unlayered thick cortex with poor cortico - medullary demarcation. On MRI ,cortex  is thick with poly microgyric and hypo myelination.

Lissencephaly type 111 shows microcephaly ,thick cortex, enlarged ventricles and hypoplastic cerebellum &  brain stem.

Reference: Diagnostic neuro-radiology by ANNE G. OSBORN.

Submitted by :DR. M. Adinarayana Rao. M.D. 

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